ALS (Exam 4)

Question 1

Amyotrophic Lateral Sclerosis

Answer 1

A relentlessly progressive - presently incurable neurodegenerative disorder that causes muscle weakness - disability - death

Question 2

ALS: Survival

Answer 2

Median = 3-5 years

10% can liver 10 years or more

Question 3

ALS: Clinical manifestations

Answer 3

Asymmetric limb weakness

Fatigue

Dysarthria / Dysphagia

Muscle atrophy

Spasticity

Fasciculations (twitch) of face or tongue

Respiratory muscle weakness

Question 4

Assymetric limb weakness

Answer 4

Most common presentation of ALS

Upper Extremity - Hand weakness or shoulder girdle muscles

Lower extremity - Begins with foot dorsiflexion

Symptoms spread within segment of onset

Question 5

What is the cause of death in ALS

Answer 5

Respiratory Muscle Weakness

Question 6

ALS illness trajectory

Answer 6

Devastating because patient remains cognitively intact while wasting away

Question 7

ALS: Drug Therapy

Answer 7

Riluzole

Not a cure but does extend survial time

Question 8

ALS: Plan of Care

Answer 8

Guide patient through moderate intensity endurance-type exercises for trunk / limbs

-reduce spasticity

-physical therapy consult

Question 9

When risk for aspiration starts it is important that we

Answer 9

maintain NPO status